Exploring sex-specific differences in the presentation and outcomes of ANCA-associated vasculitis: a nationwide registry-based cohort study

Bjørneklett, Rune; Solbakken, Vilde; Bostad, Leif; Fismen, Anne‐Siri

doi:10.1007/s11255-018-1888-8

Cited by 16 publications

(34 citation statements)

References 12 publications

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“…Although deteriorated GFR is a known predictor of ESRD [ 2 – 5 , 21 – 23 ], there are conflicting results of the effect of MPO (or p-ANCA)-positivity on the renal outcome [ 3 , 5 , 16 , 21 , 23 , 25 ]. Recent studies from Norway also related female gender to favorable renal survival in AAV [ 27 , 28 ]. The reasons for this gender-specific difference regarding ESRD risk remained elusive in our cohort and further research is required respecting this issue.…”

Section: Discussionmentioning

confidence: 99%

Prognostic Factors for Survival and Relapse in ANCA-Associated Vasculitis with Renal Involvement: A Clinical Long-Term Follow-Up Study

Salmela

Törnroth

Poussa

et al. 2018

International Journal of Nephrology

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Aim We describe the clinical pattern of ANCA-associated vasculitis (AAV) and assess long-term prognostic factors of patients and renal survival and relapse. Methods Data from 85 patients with renal biopsy-proven AAV at a single center with up to 20-year [median 16.2 years (95% CI 14.9-17.7)] follow-up were retrospectively collected. Results Overall, 55% of the patients had microscopic polyangiitis (MPA) and 45% had granulomatosis with polyangiitis (GPA). The histopathological classes were focal in 35%, crescentic in 26%, mixed in 20%, and sclerotic glomerulonephritis in 19% of the patients. As induction treatment, a combination of cyclophosphamide and corticosteroids was given to 82%, while a combination of azathioprine and corticosteroids was maintenance therapy in 79%. The twenty-year patient survival was 45%. In a multivariable analysis, age ≥58 years [hazard ratio (HR) 7.64, 95% CI 3.44-16.95] and myeloperoxidase (MPO) ANCA (HR 2.12, 95% CI 1.08-4.17) were associated with shorter patient survival time. Renal survival was 68% overall: 88% in focal, 71% in crescentic, 56% in mixed, and 37% in sclerotic class (p=0.01). Female sex (HR 0.26, 95% CI 0.10-0.73) was a predictor of improved renal survival, whereas GFR <30 ml/min and MPO-ANCA were associated with worse renal survival (HR 4.10, 95% CI 1.35-12.49 and HR 3.10, 95% CI 1.21-7.95, respectively). Relapse-free survival at 20 years was 10%. MPA was associated with a lower risk for relapse (HR 0.48, 95% CI 0.28–0.82). Conclusion We confirmed the improved patient and renal survival in AAV patients with glomerulonephritis, while relapse remained the primary challenge. Histopathological classification may be relevant for survival.

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Section: Discussionmentioning

confidence: 99%

Prognostic Factors for Survival and Relapse in ANCA-Associated Vasculitis with Renal Involvement: A Clinical Long-Term Follow-Up Study

Salmela

Törnroth

Poussa

et al. 2018

International Journal of Nephrology

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“…However, few data exist about sex-specific differences in AAV glomerulonephritis (ANCA-GN) outcomes. A recent study 14 demonstrated that males have a significantly higher risk of progression to ESKD, particularly in those with crescentic Berden class. A limitation of this study was the lack of personalised treatment data to allow modelling for association with renal outcomes.…”

Section: Introductionmentioning

confidence: 99%

“…Unlike most autoimmune diseases, AAV displays a slight male predominance 11 , 12 , with the male: female ratio reported as 1.0:0.9 13 . Proteinase 3 (PR3)-ANCA is more prevalent than myeloperoxidase (MPO)-ANCA in males 14 . However, few data exist about sex-specific differences in AAV glomerulonephritis (ANCA-GN) outcomes.…”

Section: Introductionmentioning

confidence: 99%

A cohort study to investigate sex-specific differences in ANCA-associated glomerulonephritis outcomes

Scott

Canepa

Buettner

et al. 2021

Sci Rep

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Data surrounding sex-specific differences in ANCA-associated vasculitis glomerulonephritis (ANCA-GN) outcomes is sparse. We hypothesised that the previously observed increased risk of end-stage kidney disease (ESKD) in males is driven by sex-specific variation in immunosuppression dosing. Patients were recruited to the Irish Rare Kidney Disease Registry or followed by the Royal Free Hospital vasculitis team (2012–2020). Inclusion criteria: prior diagnosis of ANCA-GN (biopsy proven pauci-immune glomerulonephritis) and positive serology for anti-MPO or -PR3 antibodies. Renal and patient survival, stratified by sex and Berden histological class, was analysed. The cumulative- and starting dose/kilogram of induction agents and prednisolone, respectively, was compared between sexes. 332 patients were included. Median follow-up was time 40.2 months (IQR 17.3–69.2). 73 (22%) reached ESKD and 47 (14.2%) died. Overall 1- and 5-year renal survival was 82.2% and 76.7% in males and 87.1% and 82.0% in females, respectively (p 0.13). The hazard ratio for ESKD in males versus females, after adjustment for age, ANCA serology, baseline creatinine and histological class was 1.07 (95% CI 0.59–1.93). There was no difference between sexes in the dose/kilogram of any induction agent. We did not observe a strong impact of sex on renal outcome in ANCA-GN. Treatment intensity does not vary by sex.

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“…In addition, sustained chronic kidney disease stage 5 is increasingly recognised as part of the ESKD de nition, but was not included in our study (in line with the Norwegian study). We chose to use identical inclusion criteria and de nitions to Bjørneklett et al 14 to enable accurate validation. Similarly, only biopsy proven cases were included, which results in selection bias towards more severe cases.…”

Section: Discussionmentioning

confidence: 99%

“…Participants of this retrospective cohort study were either previously recruited to the national Irish Rare Kidney Disease (RKD) Registry and Biobank 15 , or followed by the vasculitis team at the Royal Free Hospital (RFH), London (United Kingdom (U.K.)), between 2012-2020. Participants had to have a prior diagnosis of AAV pauci-immune glomerulonephritis, con rmed on kidney biopsy, with at least three glomeruli 5,14 , and positive serology for anti-MPO or -PR3 antibodies. Patients with secondary vasculitis or dual anti-glomerular basement membrane disease were excluded, in accordance with the European Medicines Agency algorithm 16 .…”

Section: Study Participantsmentioning

confidence: 99%

A Cohort Study to Investigate Sex-specific Differences in ANCA-associated Glomerulonephritis Outcomes

Scott

Canepa

Buettner

et al. 2021

Preprint

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IntroductionData surrounding sex-specific differences in ANCA-associated vasculitis glomerulonephritis (ANCA-GN) outcomes is sparse. We hypothesised that the previously observed increased risk of end-stage kidney disease (ESKD) in males is driven by sex-specific variation in immunosuppression dosing.MethodsPatients were recruited to the Irish Rare Kidney Disease Registry or followed by the Royal Free Hospital vasculitis team (2012-2020). Inclusion criteria: prior diagnosis of ANCA-GN (biopsy proven pauci-immune glomerulonephritis) and positive serology for anti-MPO or –PR3 antibodies. Renal and patient survival, stratified by sex and Berden histological class, was analysed. The cumulative- and starting dose/kilogram of induction agents and prednisolone, respectively, was compared between sexes. Results332 patients were included. Median follow-up was time 40.2 months (IQR 17.3–69.2). 73 (22%) reached ESKD and 47 (14.2%) died. Overall 1- and 5-year renal survival was 82.2% and 76.7% in males and 87.1% and 82.0% in females, respectively (p 0.13). The hazard ratio for ESKD in males versus females, after adjustment for age, ANCA serology, baseline creatinine and histological class was 1.07 (95% CI 0.59–1.93). There was no difference between sexes in the dose/kilogram of any induction agent.ConclusionWe did not observe a strong impact of sex on renal outcome in ANCA-GN. Treatment intensity does not vary by sex.

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Exploring sex-specific differences in the presentation and outcomes of ANCA-associated vasculitis: a nationwide registry-based cohort study

Abstract: Male sex is associated with increased risk of ESRD across all histological classes of ANCA-GN.

Cited by 16 publications

References 12 publications

Prognostic Factors for Survival and Relapse in ANCA-Associated Vasculitis with Renal Involvement: A Clinical Long-Term Follow-Up Study

Prognostic Factors for Survival and Relapse in ANCA-Associated Vasculitis with Renal Involvement: A Clinical Long-Term Follow-Up Study

A cohort study to investigate sex-specific differences in ANCA-associated glomerulonephritis outcomes

A Cohort Study to Investigate Sex-specific Differences in ANCA-associated Glomerulonephritis Outcomes

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